Epilepsy in cerebral amyloid angiopathy: an observational retrospective study of a large population

Abstract

Objective: Cerebral amyloid angiopathy (CAA) is a major cause of spontaneous intracranial hemorrhage in older adults. Epilepsy represents a possible sequela of the disease. To date, studies on epilepsy in CAA are lacking, and the few data available mainly focus on CAA-related inflammation (CAA-ri), the inflammatory form of the disease. Methods:In this retrospective observational study, we consecutively recruited CAA patients observed over a time span of 10 years, collecting demographic, clinical, and instrumental data. Significant baseline characteristics were evaluated as potential risk factors for the development of epilepsy in the CAA population, and in the subgroups of CAA-ri and CAA without inflammatory reaction (CAA-nri).The effect of potential risk factors for epilepsy was measured as odds ratio with 95% confidence interval.Results: Within 96 recruited CAA cases, 33 (34.4%) developed epilepsy during follow-up (median = 13.5 months). The prevalent type of seizure was focal (81.3%); 12.1% of the epileptic patients presented status epilepticus, and 6.1% developed drug-resistant epilepsy. Electroencephalographic traces revealed slow and epileptic discharge activity in the majority of epileptic patients, but also in those without epilepsy. The presence of focal or disseminated cortical superficial siderosis (cSS) was associated with an increased risk of epilepsy in the CAA-nri group, and the association with CAA-ri and epilepsy was present in the overall population.Significance: Epilepsy is a common manifestation during the course of CAA, where CAA-ri and cSS represent predisposing factors for the development of seizures. These data suggest the importance of a deep characterization of CAA patients, to better select those more prone to develop epilepsy.

Incidence and risk factors associated with seizures in cerebral amyloid angiopathy

“…A recent retrospective study from Tabaee et al reported 33 of 96 (33.3%) patients with CAA were diagnosed with epilepsy during a 13.5‐month follow‐up period [31]. Their report compares to our findings with CAA‐ri and superficial siderosis correlated with a higher incidence of seizures.…”

“…However, the difference was not statistically significant, likely due to the small size of the study cohort and lower incidence of seizures [19]. Our study includes one of the largest cohorts of patients with and without CAA‐ri [31, 45], and supports inflammation associated with CAA as a risk factor for seizures and epilepsy.…”

The Winners of the Cerebral Amyloid Angiopathy Epilepsy Prize Are: Blood and Inflammation

“…In the current study, 4 a cohort of retrospectively identified patients diagnosed with CAA over a 10-year period in a single Center, San Gerardo, in Italy was identified. The diagnosis of CAA was made based on the newly released Boston 2.0 criteria 5 which rely heavily on MRI features suggestive of CAA.…”

Zerebrale Mikroangiopathie: Fortschritte im Verständnis der Pathophysiologie

“…1). Gemäß einer kürzlich publizierten retrospektiven Beobachtungsstudie, erkranken etwa 1/3 aller CAA-Patienten an einer Epilepsie; 80 % haben fokale epileptische Anfälle [18]. Vergleichbar zur pathophysiologischen und MRT-basierten Stadienhaftigkeit der Mikroangiopathie (siehe unten), wird zumindest bei der CAA auch eine Stadienhaftigkeit des Phänotyps diskutiert: kognitive Defizite kennzeichnen wahrscheinlich ein früheres, große Hirnblutungen ein späteres Krankheitsstadium [19].…”